Multiple recurrent aneurysms with angiitis of the central nervous system in a girl: A case report

Wen, Jiayu; Ye, Shengda; Yang, Bin; Liu, Xi; Chen, JinCao

doi:10.1097/md.0000000000032415

Cited by 3 publications

(2 citation statements)

References 8 publications

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“…Unruptured intracranial aneurysms have been described uncommonly in PCNSV (7–10). In our series, UIA was detected at diagnosis or during follow-up in 12 of 216 patients (5.5%) with PCNSV.…”

Section: Discussionmentioning

confidence: 99%

“…Although characterized by a limited specificity, angiographic findings suggesting a high probability of vasculitis include areas of smooth-wall segmental narrowing or dilation and occlusions that affect multiple cerebral arteries without proximal vessel changes of atherosclerosis or other possible causes (1,3,4). Intracranial aneurysms are rarely reported in PCNSV (7)(8)(9)(10).…”

Section: Conclusion Uias Uncommonly Occur In Pcnsvmentioning

confidence: 99%

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Primary Central Nervous System Vasculitis With Intracranial Aneurysm

Salvarani,

Brown,

Christianson

et al. 2024

Preprint

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Background. Unruptured intracranial aneurysms (UIAs) are rarely reported in primary central nervous system vasculitis (PCNSV). In this study we described the clinical findings, response to therapy, and outcomes of UIA in a large cohort of PCNSV patients. Methods. We retrospectively studied 216 consecutive patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 40-year period. UIAs were identified on cerebral angiography. The clinical, laboratory, radiologic and pathologic findings, management, and outcomes of patients with UIA were described and compared with those without UIA. Results. 12/216 (5.5%) PCNSV patients had at least one UIA. In the only positive patient biopsy showed necrotizing vasculitis. Eleven patients had evidence of UIA on angiogram at diagnosis. One patient developed an aneurysm during the follow-up associated with a worsening of vasculitic radiological findings. The most common presenting symptom for PCNSV in the setting of UIA was headache (67%), followed by persistent neurologic deficit or stroke (50%). Most patients with UIA presented with multiple cerebral infarcts on MRI (67%), one patient had subarachnoid hemorrhage, and one left parieto-occipital intracerebral hematoma, both unrelated to the aneurysm. Black blood imaging was performed in 4 patients and 2 showed segmental circumferential mural enhancement involving multiple vessels. Two patients had 2 UIAs, while the other 10 had 1. The most frequent UIA location was internal carotid artery (50%), followed by anterior cerebral artery (21%). Ten of the UIAs were < 5 mm in diameter, and 3 were 5-7 mm in diameter; the size was not available for one. All UIAs were unchanged in size and configuration during follow-up and no new aneurysms were detected. Compared to the 204 patients with PCNSV without a UIA, no significant clinical differences were observed, except for a reduced disability at last follow-up (p = 0.038). Conclusions. UIAs uncommonly occur in PCNSV.

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Section: Discussionmentioning

confidence: 99%